Summary
Cluster headache (CH) is a type of primary headache that mostly affects adult men. Patients present with recurrent, fifteen minute up to three hour attacks of agonizing, strictly unilateral headaches in the periorbital and forehead region (areas innervated by the trigeminal nerve). These attacks are associated with ipsilateral symptoms of increased cranial autonomic activity, e.g., lacrimation, conjunctival injection, rhinorrhea, or partial Horner syndrome. Cluster headaches tend to occur in episodic patterns (“cluster bouts”) followed by months of remission, but are considered chronic if remission between bouts lasts less than one month. Diagnosis is based on the patient's history, in particular on the exact description and timing of the headaches. Acute episodes are treated with 100% oxygen or triptans, while verapamil is used for preventative treatment.
Epidemiology
References:[1]
Epidemiological data refers to the US, unless otherwise specified.
Etiology
- Not entirely understood
- Risk factor: tobacco use
- Possible triggers: alcohol, histamine, seasonal fluctuations
References:[1][2][3]
Clinical features
- Headache characteristics
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Ipsilateral autonomic symptoms
- Conjunctival injections and/or lacrimation
- Rhinorrhea and nasal congestion
- Partial Horner syndrome: ptosis and miosis, but no anhidrosis
- Restlessness and agitation
While patients with migraine headaches tend to rest motionlessly in a quiet, dark room, individuals with cluster headache pace around restlessly in excruciating pain!
References:[1][2][4][5][6][7]
Diagnostics
- Based on patient history and physical examination
- Rule out any suspected underlying disease
References:[2]
Differential diagnoses
- See article on “Headache” for more information regarding differential diagnoses.
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Paroxysmal hemicrania
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Epidemiology
- Rare; the exact prevalence is unknown
- Equal gender distribution: ♀ = ♂ [8]
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Clinical features
- Severe unilateral attacks of periorbital pain that recur several times per day
- Cranial autonomic dysfunction (e.g., lacrimation, conjunctival injection)
- Attacks are generally more frequent (usually ≥ 5 per day) and shorter (2–45 min) than cluster headaches and may occur at any time of the day.
- Treatment: no established treatment for acute attacks
- Prevention: indomethacin
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Epidemiology
-
Short-lasting unilateral neuralgiform headache attacks
- Definition: a form of trigeminal autonomic cephalgia characterized by unilateral throbbing pain and autonomic symptoms (i.e., conjunctival injection and/or tearing)
- Subtypes
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Clinical features
- Unilateral throbbing, piercing, and/or burning head pain with temporal, orbital, or supraorbital distribution
- Attacks are frequent
- At least 1 per day
- Last between 1 second and 10 minutes as single stabs, a series of stabs, or in a saw-tooth pattern
- At least one cranial autonomic symptom is usually present
- Conjunctival injection and/or lacrimation (tearing)
- Eyelid edema
- Facial and forehead sweating
- Miosis and/or ptosis
- Nasal congestion and/or rhinorrea
- Attacks can be triggered without a refractory period
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Diagnosis: mainly clinical following a history of atleast 20 attacks; CT or MRI may be used to rule out other causes (e.g., tumor)
- SUNCT is diagnosed if both conjunctival injection AND lacrimation are present ipsilateral to the pain
- SUNA is diagnosed if not > 1 of conjunctival injection or lacrimation occurs ipsilateral to the pain
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Treatment: no abortive treatment
- Acute attacks: IV lidocaine decreases frequency of attack.
- Prevention: antiepileptic drugs (e.g., lamotrigine, topiramate, or gabapentin)
References:[9][10][11][12][13][14]
The differential diagnoses listed here are not exhaustive.
Treatment
Medical therapy
-
Acute
- Oxygen therapy with FiO2 100%
- First-line: : triptans (e.g., sumatriptan) or zolmitriptan
- Pain relievers (i.e. NSAIDs) are generally not recommended because their onset of action is too slow.
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Prevention
-
First-line treatment: verapamil
- Steroids (e.g., prednisone) are very effective at ending a cluster cycle and may be used to bridge the time until verapamil becomes effective.
- Second-line treatment: lithium, topiramate, ergot derivatives
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First-line treatment: verapamil
Interventional therapy
Interventional procedures (e.g., ablative injections, deep brain stimulation) may be considered in patients with cluster headache who do not respond to medical therapy.
References:[2][15][16]
Acute management checklist
- Consider obtaining an MRI of the brain with and without contrast to rule out secondary causes in patients with new diagnosis of cluster headache. [17][18][19]
- Consult neurology.
- Start abortive therapy. [17][19][20][21]
- First-line
- 100% supplemental oxygen (e.g., 6–12 L/min via nonrebreather face mask in an upright position) [22]
- Sumatriptan
- Zolmitriptan
- Alternative therapies:
- First-line
- Start prophylactic therapy
- Consider admission to neurologic service for patients with their first episode of cluster headache, suicidal ideation, or refractory pain.
Apply nasal sprays in the nostril that is not affected by congestion to improve absorption (except lidocaine, which acts locally).
Ergotamines and triptans should not be taken within 24 hours of one another because of the risk of coronary artery spasms.
Ischemic vascular disease, accessory conduction pathway disorders, and pregnancy are important contraindications for triptans and ergotamines.